CSC Congenital Heart Disease Surgery 3

Question 1

The Rastelli procedure is specifically designed to repair which combination of congenital defects?

Accepted Answer

D-TGA with VSD and left ventricular outflow tract obstruction

Answer

Hypoplastic left heart syndrome with aortic atresia

Answer

Tricuspid atresia with normally related great arteries

Answer

Tetralogy of Fallot with absent pulmonary valve syndrome

Question 2

What is the primary long-term consequence of leaving a large, unrestrictive VSD untreated beyond early childhood?

Accepted Answer

Eisenmenger syndrome with irreversible pulmonary vascular obstructive disease

Answer

Spontaneous closure with formation of a ventricular aneurysm

Answer

Progressive right ventricular pressure overload causing right heart failure

Answer

Development of complete atrioventricular block from septal fibrosis

Question 3

In a complete atrioventricular septal defect (AVSD), how many leaflets does the common AV valve typically contain?

Accepted Answer

Five leaflets spanning both the tricuspid and mitral annuli

Answer

Two leaflets with a single central orifice

Answer

Three leaflets functioning exclusively as a right-sided valve

Answer

Four leaflets with a secundum ASD and perimembranous VSD

Question 4

Which chromosomal abnormality is most strongly associated with complete atrioventricular septal defect?

Accepted Answer

Trisomy 21 (Down syndrome)

Answer

Turner syndrome (45, X)

Answer

22q11.2 deletion (DiGeorge syndrome)

Answer

7q11.23 deletion (Williams syndrome)

Question 5

The Konno-Rastan procedure (aortoventriculoplasty) is indicated for which condition?

Accepted Answer

Severe tunnel-type subaortic stenosis with an annulus too small for adequate prosthesis

Answer

Subaortic membrane with a mild 20 mmHg gradient

Answer

Supravalvular aortic stenosis associated with Williams syndrome

Answer

Bicuspid aortic valve with moderate stenosis and adequate annulus size

Question 6

What is the primary purpose of pulmonary artery banding as a palliative procedure in congenital heart surgery?

Accepted Answer

To restrict excessive pulmonary blood flow and protect pulmonary vasculature from hypertensive injury

Answer

To augment pulmonary blood flow in cyanotic heart defects

Answer

To create a controlled atrial-level communication for mixing

Answer

To provide systemic blood flow when the ductus arteriosus closes

Question 7

During surgical repair of supracardiac total anomalous pulmonary venous connection, what is the critical anastomosis performed?

Accepted Answer

Wide anastomosis between the posterior common pulmonary venous confluence and the posterior left atrium

Answer

Ligation of the vertical vein with direct individual pulmonary vein reimplantation

Answer

Connection of the innominate vein directly to the left atrial appendage

Answer

Creation of a baffle directing pulmonary venous blood through the coronary sinus to the left atrium